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Classification

Authoring team

A pathological classification of porphyrias concentrates on the site of the defect in haem synthesis:

  • defects in the liver cause hepatic porphyrias
  • defects in the marrow cause erythropoietic porphyrias

A clinically more useful classification is the division in to acute and non-acute porphyrias:

  • acute poryphrias typically present with abdominal pain and neuropsychiatric crises
  • non-acute porphyrias cause photosensitivity

Reference

  1. Karim Z, Lyoumi S, Nicolas G, et al; Porphyrias: A 2015 update. Clin Res Hepatol Gastroenterol. 2015 Jul 1. pii: S2210-7401(15)00120-5.

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